Immunologic Studies in von Willebrand's Disease: EVIDENCE THAT THE ANTIHEMOPHILIC FACTOR (AHF) PRODUCED AFTER TRANSFUSIONS LACKS AN ANTIGEN ASSOCIATED WITH NORMAL AHF AND THE INACTIVE MATERIAL PRODUCED BY PATIENTS WITH CLASSIC HEMOPHILIA

AUTOR(ES)

Bennett, Bruce

RESUMO

Antihemophilic globulin (AHF, factor VIII) levels were measured by a standard coagulation assay and by an immunological technique before and serially after infusion of fresh frozen plasma or cryoprecipitate into patients with von Willebrand's disease. Initial levels of AHF, measured both as procoagulant and as antigen, were low. Immediately after transfusions, the rise in levels of AHF-like antigen was compatible with the quantity of antigen present in the infused plasma or cryoprecipitate. Thereafter, levels of antigen declined rapidly and reached preinfusion values in approximately 24 hr. In contrast, procoagulant activity remained elevated, and sometimes continued to rise, for longer periods of time. One possible explanation of this finding is that the AHF molecule produced by patients with von Willebrand's disease, in response to transfusion of as yet unidentified factors, lacks the antigenic site associated with the normal AHF molecule or the inactive molecule produced by patients with hemophilia A.

Documentos Relacionados

• Immunologic differentiation of classic hemophilia (factor VIII deficiency) and von Willebrand's disease: With observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor
• von Willebrand's disease antigen II. A new plasma and platelet antigen deficient in severe von Willebrand's disease.
• Studies of the human factor VIII/von Willebrand factor protein. III. Qualitative defects in von Willebrand's disease.
• Carbohydrate of the Factor VIII/von Willebrand Factor in von Willebrand's Disease
• Detection of carriers of classic hemophilia using an immunologic assay for antihemophilic factor (factor VIII)